Abstract
A 2-year-4-month-old boy with sudden onset of abdominal pain was referred to our hospital. He was diagnosed as having ruptured hepatoblastoma with tumor thrombosis in the main portal vein (PRETEXT IV). Even though he received neoadjuvant chemotherapy (CITA × 2, ITEC × 1), the tumor thrombosis remained in the main trunk of the portal vein up to the bilateral portal branches. After the fourth course of chemotherapy by CDDP, a living-donor liver transplantation using a lateral segment liver graft from his mother was performed for the total resection of the unresectable hepatoblastoma. Intraoperative findings showed that the bilateral portal vein was filled with the tumor thrombus extending into the main portal trunk. Rapid intraoperative histopathological analysis revealed tumor-cell-negative surgical margins of the portal vein, and complete resection was achieved. Four courses of postoperative chemotherapy were performed and he has been alive without recurrence for five years and ten months after liver transplantation. Although the combination of liver transplantation and total hepatectomy for hepatoblastoma with venous invasion is controversial, excellent outcome can be obtained if the invaded venous structures are completely resected.
